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[Research progress of bile acid spectrum in hereditary deficiency of bile acid synthesis]
- Source :
- Zhonghua er ke za zhi = Chinese journal of pediatrics. 60(8)
- Publication Year :
- 2022
-
Abstract
- 胆汁酸在促进脂质、脂溶性维生素吸收和维持正常肠肝循环方面起重要作用。由胆固醇合成胆汁酸至少需要经过17种酶的催化反应，任何1种酶缺陷都会造成胆汁酸合成障碍，导致血浆和（或）尿液中疾病特异性胆汁酸中间产物的积累，引起脂溶性维生素吸收不良、胆汁淤积性肝病及神经系统病变等症状。通过串联质谱分析技术检测血浆、尿液中的胆汁酸谱是协助遗传性胆汁酸合成缺陷症诊断、判断疾病严重程度和预后的简便而有效的手段。.
- Subjects :
- Bile Acids and Salts
Liver
Humans
Subjects
Details
- ISSN :
- 05781310
- Volume :
- 60
- Issue :
- 8
- Database :
- OpenAIRE
- Journal :
- Zhonghua er ke za zhi = Chinese journal of pediatrics
- Accession number :
- edsair.pmid..........fea1068cbb6b6bbf25a1d3f084a1c76f